Mast cell leukemia

Source: Wikipedia, the free encyclopedia.
Acute mast cell leukemia
Peripheral blood showing mast cell leukemia.
SpecialtyHematology and oncology

Mast cell leukemia is an extremely aggressive subtype of

mast cells in marrow and 10% in blood.[1] If the mast cells
represent less than 10% of blood cells, the tumor is called "aleukemic" mast cell leukemia.

Signs and symptoms

Acute mast cell leukemia is a rapidly progressive disorder with leukemic mast cells in blood and in large numbers in marrow. The common signs and symptoms include fever, headache,

computerized tomography (CT) scanning is used to look for hepatosplenomegaly and lymphadenopathy. Plain radiography and bone densitometry can be used to assess bone involvement and the presence of osteoporosis. Endoscopy and biopsy can be useful if gut involvement is suspected.[8]

Diagnosis

Cytochemistry

Cytochemical properties of the leukemic cells must be typical of mast cell derivation (presence of

metachromatic granules staining with alpha-naphthyl chloroacetate esterase, but not with peroxidase).[6] Mast cell tryptase is an enzyme contained in mast cell granules. Mast cell numbers are best estimated by tryptase immunostaining because very poorly granulated cells may stain very weakly if at all for alpha-naphthol chloroacetate esterase.[1]

Tumor markers

The leukemic cells usually are strongly positive for

CD25.[9] Malignant mast cells overexpress the anti-apoptosis gene, bcl-2.[10] A mutation called KIT mutation is detected in most patients.[11]

Biochemistry

Total serum tryptase is elevated in mast cell leukemia. Normal total (alpha + beta) serum tryptase is approximately 6 micro g/L (range 0 to 11 micro g/L). Values of several hundred micro g/L are characteristic of mast cell leukemia.[12] Plasma and urinary histamine levels are frequently elevated in mast cell leukemia. Histidine decarboxylase (HDC) is the enzyme that catalyzes the reaction which produces histamine from histidine. Measurement of histidine carboxylase in the marrow cells of patients with mast cell leukemia is a very sensitive marker of mast cells.[13]

Treatment

Immunoglobulin E (

Stem cell transplantation is an option, although no experience exists concerning responses and outcome.[11]

Prognosis

Acute mast cell leukemia is extremely aggressive and has a grave prognosis. In most cases, multi-organ failure including bone marrow failure develops over weeks to months.[16] Median survival after diagnosis is only about 6 months.[4]

References

  1. ^
    PMID 16203163
    .
  2. PMID 54900
    .
  3. PMID 9739925
    .
  4. ^
    PMID 3095598
    .
  5. PMID 11377685
    .
  6. ^ a b Kufe D, et al. (2000). Holland Frei Cancer Medicine (5th ed.). BC Decker.
  7. PMID 16389403
    .
  8. ^ Hoffbrand AV, Catovsky D, Tuddenham E (2005). Postgraduate Haematology (5th ed.). Blackwell.
  9. S2CID 23872236
    .
  10. PMID 10072109
    .
  11. ^ a b Ansell SM, ed. (2008). Rare Hematological Malignancies. Cancer Treatment & Research. Springer.
  12. S2CID 42677668
    .
  13. PMID 15562525
  14. PMID 8157759
    .
  15. S2CID 9763026
    .
  16. ^ Hoffman R, Benz E, Shattil S, Furie B, Cohen H (2004). Hematology: Basic Principles and Practice (4th ed.). Churchill Livingstone.

External links

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